Obiettivo Nuclease Immune Mediated Brain and Lupus-like (NIMBL) conditions, comprising Aicardi-Goutieres Syndrome, Retinal Vasculopathy with Cerebral Leukodystrophy and some cases of Systemic Lupus Erythematosus, are devastating genetic disorders resulting in greatly reduced quality of life, high mortality especially in children, and significant risks of recurrence within affected families. NIMBL conditions are rare, but under diagnosed. No effective treatments or cures exist at present. To enable optimum patient care in Europe and worldwide, a better understanding of the natural course of these disorders and their underlying pathological basis is essential. In the NIMBL project, European and U.S. clinical and basic scientists at the forefront of NIMBL-related research have united to develop a translational approach to these problems. A registry of patients will reveal the natural history of the NIMBL diseases, and the efficacy of current treatments. Investigation of patients together with the use of existing and novel cellular and animal models will precisely define their pathogenesis and identify potential drug targets. These same platforms will then be used to test existing and new drug treatments, thus providing unique opportunities to European companies. The NIMBL project will build on very recent discoveries of the cell-intrinsic initiation of autoimmunity that we have described, and which have major implications for our understanding of the discrimination of self from non-self. This new biological paradigm involves cytosolic sensors that detect accumulated, endogenous nucleic acids, and induce the body to mount an immune response against its own cells. Thus, the investigation of NIMBL diseases will not only improve the health and well-being of NIMBL patients and their families, it will also lead to better treatments of much more common autoimmune disorders including lupus. Campo scientifico natural sciencesbiological sciencesbiochemistrybiomoleculesnucleic acidshumanitieshistory and archaeologyhistorymedical and health sciencesbasic medicinephysiologypathophysiologysocial sciencessociologysocial issuessocial inequalitiesmedical and health sciencesbasic medicineimmunology Programma(i) FP7-HEALTH - Specific Programme "Cooperation": Health Argomento(i) HEALTH-2009-2.4.4-1 - Rare neurological diseases Invito a presentare proposte FP7-HEALTH-2009-single-stage Vedi altri progetti per questo bando Meccanismo di finanziamento CP-FP - Small or medium-scale focused research project Coordinatore THE UNIVERSITY OF MANCHESTER Contributo UE € 1 379 436,00 Indirizzo OXFORD ROAD M13 9PL Manchester Regno Unito Mostra sulla mappa Regione North West (England) Greater Manchester Manchester Tipo di attività Higher or Secondary Education Establishments Contatto amministrativo Liz Fay (Ms.) Collegamenti Contatta l’organizzazione Opens in new window Sito web Opens in new window Costo totale Nessun dato Partecipanti (8) Classifica in ordine alfabetico Classifica per Contributo UE Espandi tutto Riduci tutto ACADEMISCH ZIEKENHUIS LEIDEN Paesi Bassi Contributo UE € 620 162,00 Indirizzo ALBINUSDREEF 2 2333 ZA Leiden Mostra sulla mappa Regione West-Nederland Zuid-Holland Agglomeratie Leiden en Bollenstreek Tipo di attività Higher or Secondary Education Establishments Contatto amministrativo Linda Ouwerkerk (Ms.) Collegamenti Contatta l’organizzazione Opens in new window Sito web Opens in new window Costo totale Nessun dato FONDAZIONE ISTITUTO NEUROLOGICO NAZIONALE CASIMIRO MONDINO Italia Contributo UE € 222 489,00 Indirizzo Via Mondino 2 27100 Pavia Mostra sulla mappa Regione Nord-Ovest Lombardia Pavia Tipo di attività Research Organisations Contatto amministrativo Andrea Marchetti (Dr.) Collegamenti Contatta l’organizzazione Opens in new window Sito web Opens in new window Costo totale Nessun dato UNIVERSITY OF LEEDS Regno Unito Contributo UE € 748 642,00 Indirizzo WOODHOUSE LANE LS2 9JT Leeds Mostra sulla mappa Regione Yorkshire and the Humber West Yorkshire Leeds Tipo di attività Higher or Secondary Education Establishments Contatto amministrativo Ben Williams (Mr.) Collegamenti Contatta l’organizzazione Opens in new window Sito web Opens in new window Costo totale Nessun dato ACADEMISCH MEDISCH CENTRUM BIJ DE UNIVERSITEIT VAN AMSTERDAM Paesi Bassi Contributo UE € 672 947,00 Indirizzo MEIBERGDREEF 15 1105AZ Amsterdam Mostra sulla mappa Regione West-Nederland Noord-Holland Groot-Amsterdam Tipo di attività Higher or Secondary Education Establishments Contatto amministrativo Sharon Rieskin (Ms.) Collegamenti Contatta l’organizzazione Opens in new window Sito web Opens in new window Costo totale Nessun dato FUNDACIO INSTITUT DE RECERCA BIOMEDICA (IRB BARCELONA) Partecipazione conclusa Spagna Contributo UE € 518 651,41 Indirizzo CARRER BALDIRI REIXAC 10-12 PARC SCIENTIFIC DE BARCELONA 08028 Barcelona Mostra sulla mappa Regione Este Cataluña Barcelona Tipo di attività Research Organisations Contatto amministrativo Alexandre Puerto Valdivieso (Mr.) Collegamenti Contatta l’organizzazione Opens in new window Sito web Opens in new window Costo totale Nessun dato UNIVERSITY OF WASHINGTON Stati Uniti Contributo UE € 667 893,00 Indirizzo BROOKLYN AVENUE NE 4333 98195 9472 Seattle Wa Mostra sulla mappa Tipo di attività Higher or Secondary Education Establishments Contatto amministrativo Lynne Chronister (Ms.) Collegamenti Contatta l’organizzazione Opens in new window Sito web Opens in new window Costo totale Nessun dato Children's Research Institute (CRI) Stati Uniti Contributo UE € 357 444,00 Indirizzo Michigan Avenue 111 20010 Washington, DC Mostra sulla mappa Tipo di attività Research Organisations Contatto amministrativo Monique Foxx (Ms.) Collegamenti Contatta l’organizzazione Opens in new window Costo totale Nessun dato UNIVERSITAT DE BARCELONA Spagna Contributo UE € 209 328,59 Indirizzo GRAN VIA DE LES CORTS CATALANES 585 08007 Barcelona Mostra sulla mappa Regione Este Cataluña Barcelona Tipo di attività Higher or Secondary Education Establishments Contatto amministrativo Xavier Gutierrez (Mr.) Collegamenti Contatta l’organizzazione Opens in new window Sito web Opens in new window Costo totale Nessun dato